Effect of late postoperative atrial septal defect closure on hemodynamic function in patients with a Lateral tunnel Fontan procedure.

OBJECTIVES: The aim of this study was to evaluate prospectively the effect of late atrial septal defect closure on cardiac output and oxygen delivery in patients who have undergone the Fontan procedure. BACKGROUND: An adjustable atrial septal defect is incorporated in patients undergoing the Fontan procedure who have increased pulmonary vascular resistance or poor ventricular function, or both. After the Fontan procedure, the atrial septal defect is test occluded. Patients with mean right atrial and pulmonary artery pressures > 15 mm Hg are discharged with the atrial septal defect open. METHODS: Twelve patients (20 months to 12 years old) underwent evaluation and closure of the atrial septal defect at a mean interval of 3.8 months (range 1 to 18) after the Fontan procedure. Each patient underwent full right and left heart catheterization. Cardiac output was obtained using the cine-volume method. The study included six patients with a high transpulmonary gradient or poor ventricular function preoperatively, or both (high risk group) and six who had only borderline increased pulmonary vascular resistance (low risk group). Patients in both groups had a mean right atrial pressure > 15 mm Hg when the atrial defect was test occluded in the first week after the Fontan procedure. RESULTS: All results are given as mean value +/- SD. Ventricular end-diastolic pressure was significantly lower (p = 0.03) with the atrial septal defect open in low risk patients (6 +/- 3 mm Hg) than in high risk patients (10 +/- 3 mm Hg). With the atrial septal defect open, low risk patients had a significantly higher (p = 0.04) cardiac index (4.87 +/- 0.81 liters/min per m2) than the high risk patients (3.96 +/- 0.47 liters/min per m2). There was no significant difference (p = 0.14) in cardiac index between the two groups with occlusion of the atrial septal defect. Oxygen delivery was also significantly higher (p < 0.05) with the atrial septal defect open in low risk patients (836 +/- 99 ml/min per m2) than in high risk patients (704 +/- 106 ml/min per m2). There was no significant difference (p = 0.89) in oxygen delivery between the two groups with occlusion of the atrial septal defect. With the atrial septal defect open, the interatrial gradient was not significantly different in low risk patients (4 +/- 1 mm Hg) from that in high risk patients (4 +/- 1 mm Hg). CONCLUSIONS: These data show that an interatrial communication results in increased postoperative systemic perfusion and oxygen delivery in patients with good diastolic ventricular function after the Fontan procedure.

Factors influencing survival in patients undergoing the bidirectional Glenn anastomosis.

The bidirectional Glenn anastomosis (BGA) has long been used as a surgical intervention for patients with single ventricle physiology. Initially, this procedure was the final stage in palliation and was performed in older children. Eventually, as the Fontan procedure came to be used as a method to separate circulations, the Glenn procedure was performed as an intermediate step. Over time, the BGA was performed as an alternative for patients who were considered to be at high risk with the Fontan procedure. Between January 1, 1988, and January 1, 1994, 129 patients underwent BGA at the University of California-Los Angeles. These patients were reviewed retrospectively, including clinic visits, catheterization, and echocardiographic information. The overall survival rate was 87% (112 of 129 patients). The average length of follow-up was 27 months. This information was then analyzed by univariate and multivariate analysis. Several factors were related to failure in patients who underwent BGA including pulmonary artery pressure, systemic right ventricle, and presence of anomolous pulmonary venous drainage and heterotaxy syndrome.

Selective anterograde coronary arteriography in neonates with d-transposition of the great arteries: accuracy and safety.

OBJECTIVES: This study was designed to evaluate the accuracy and safety of selective anterograde coronary arteriography for the identification of the origin and branching pattern of the three main coronary arteries in neonates with d-transposition of the great arteries. BACKGROUND: Definition of coronary artery anatomy is important in neonates with d-transposition of the great arteries who are considered for the arterial switch operation. Balloon occlusion aortography defines coronary artery anatomy in most but not all cases. We have described a technique for selective anterograde coronary arteriography. METHODS: Between March 1987 and May 1991, 17 neonates underwent selective anterograde coronary arteriography and 29 patients had balloon occlusion aortography. After venous access was gained, a mesenteric catheter was used to engage the coronary ostia for contrast injection. All angiograms were reviewed by three independent observers and the coronary artery diagnoses were compared with operative findings. Complications with the catheterization procedure were also recorded. RESULTS: The accuracy of defining coronary artery anatomy with selective anterograde coronary arteriography (98 +/- 2%) was significantly (p < 0.05) greater than that achieved with balloon occlusion aortography (69 +/- 6%). There were no deaths with catheterization in either study group. Morbidity was similar in the groups with balloon occlusion aortography (7%) and selective anterograde coronary arteriography (6%) (p > 0.05) and was related to transient bradycardia induced by catheter manipulation in the right ventricle. No patient in either study group experienced cardiac ischemia. CONCLUSIONS: Selective anterograde coronary arteriography is an accurate and safe technique for the definition of coronary artery pattern in neonates with d-transposition of the great arteries.

Description of a venous technique for selective coronary arteriography in newborns with d-transposition of the great arteries.

An accurate description of the coronary anatomy is desired before anatomic correction of d-transposition of the great arteries. Two-dimensional echocardiography and aortic root angiography are occasionally unable to delineate the coronary arteries. In neonates, the arterial approach for selective coronary arteriography is difficult and may compromise visceral and lower limb perfusion. A venous technique was used in two newborns to define selectively the coronary origins and their distribution. The venous approach for selective coronary arteriography may be effective in the evaluation of newborns with d-transposition of the great arteries when echocardiography inadequately defines the coronary circulation.

Ventricular and pulmonary artery volumes in patients with absent pulmonary valve. Factors affecting the natural course.

Right and left ventricular (RV and LV) volumes were determined in 19 patients with absent pulmonary valve syndrome using Simpson's rule and area-length methods. The volume of the proximal right pulmonary artery (RPAV) was calculated at maximal and minimal size using the area-length method. Patient groups included four newborns who responded to medical management (group 1A), seven critically ill newborns who died (group 1B), four infants ages 1-10 months (group 2) and four children ages 2-8 years (group 3). The RV end-diastolic volume in groups 1B and 2 was significantly greater than that in groups 1A and 3. The RV ejection fraction in groups 1B and 2 was significantly less than that in normal patients and groups 1A and 3. Maximal RPAV correlated well with RV stroke volume and end-diastolic volume. The maximal RPAV in group 1B was significantly greater than that in groups 1A and 3. Pulmonary arterial compliance was greater than normal in all groups, and the compliance in group 1B was more than two times that in the other groups. We conclude that the increased right pulmonary artery compliance and pulmonary regurgitation in patients with absent pulmonary valve contribute to bronchial obstruction and right-heart failure and are the causes of the high morbidity and mortality in these patients. The management should be directed to the alleviation of bronchial obstruction and right-heart failure.

Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome.

The most common cardiac defect in Turner syndrome has been described previously as coarctation of the aorta. We have evaluated 35 consecutive patients with Turner syndrome by clinical examination and by M-mode and two-dimensional echocardiography. Twelve patients (34%) had isolated, nonstenotic bicuspid aortic valve. A high correlation (82%) existed between the presence of a systolic ejection click and echocardiographic evidence of a bicuspid aortic valve. These data indicate that bicuspid aortic valve may be the most common cardiac anomaly in Turner syndrome.

Two-dimensional echocardiographic assessment of right ventricular volume in children with congenital heart disease.

The ability of 2-dimensional echocardiography to measure right ventricular (RV) volume and ejection fraction was assessed in 22 children with congenital heart disease. From the apical 4 chambers 2-dimensional echocardiographic image, the long-axis length of the right ventricle was measured and the area planimetered. On the anteroposterior and lateral cineangiocardiographic planes, the right ventricle was separated into 2 parts: RV sinus and outflow tract. The longest length, inflow tract length, and area of the sinus were measured from biplane cineangiographic views. The echographic long-axis length correlated well with the longest length of the RV sinus measured from both anteroposterior and lateral cineangiographic views at both end-systole and end-diastole. Moreover, the echographic area correlated well with the sinus area obtained from both cineangiographic views. From these regression analyses, the echographic long axis length and area were corrected to the angiographic longest length and area of the sinus. The new corrected echographic longest length and area were applied to 3 formulas (2 biplane and 1 uniplane) to calculate the sinus volume of the right ventricle. Total RV volume was then derived from the sinus volume. RV volumes and ejection fraction determined by 2-dimensional echocardiography were compared with those obtained from biplane cineangiography using Simpson's rule method. All formulas tested predicted RV volumes and ejection fraction with equal accuracy. Thus, 2-dimensional echocardiography can assess RV volume and ejection fraction in children with congenital heart disease.

Two-dimensional echocardiographic evaluation of discrete subaortic stenosis from the apical long axis view.

M-mode standard two-dimensional (2DE) left parasternal long axis echocardiographic examination of the left ventricular outflow tract (LVOT) was evaluated exclusively with respect to its utility in identifying discrete subaortic stenosis (SUB-AS). Important details of the anatomy of the subaortic area may also be obtained from 2DE apical long axis imaging. According, 18 patients with discrete SUB-AS were prospectively evaluated by M-mode and 2DE. The M-mode findings included narrowing of the LVOT and early systolic closure of the aortic valve. However, these findings were variable and highly dependent upon scan speed, fluid flow dynamics, and beam angulation. 2DE findings varied using the standard long axis view at the left parasternal border, depending upon the type of obstruction present. A discrete membrane produced linear echoes adjacent and parallel to the interventricular septum beneath the aortic valve. Fibromuscular obstruction produced a localized dense ridge of echoes in the LVOT. These findings were not apparent in five patients studied. In these patients, the 2DE apical long axis view was employed to image the subaortic area. From this tomographic corss-section a fibrous membrane was imaged as a linear echo parallel to the aortic valve. The membrane extended across the LVOT from the ventricular septum to the anterior leaflet of the mitral valve. The 2DE apical long axis view therefore provides an additional approach in the evaluation of patients with discrete SUB-AS.

Ultrasonography of mucocutaneous lymph node syndrome.

Clinical findings associate mucocutaneous lymph node syndrome (MCLS) with diffuse arteritis leading to the formation of large aneurysms. Ultrasound assists in the diagnosis of the associated aneurysms, and aids the clinical management of the patients.

Accuracy of echocardiography in assessing left ventricular dimensions and volume.

The accuracy of determining left ventricular function from echocardiography was assessed in 26 children (group I) with cineangiographically-determined normal left ventricular volume (LVV) and 28 children (group II) with large left ventricular volumes. Conventional LV echo dimensions were compared to the cineangiographic LV anterior-posterior minor axis (LVmA) and LVV. Very good correlations were found in group I between LV end-diastolic echo dimensions (LVEDD) and cine LVmA (r = 0.91) and between LVEDD and LV end-diastolic volume (LVEDV) by cine in group I (r = 0.86). In group II correlations were less accurate between LVEDD and diastolic LVmA and between LVEDD and LVEDV. There was poor correlation between the cine and echo percent of shortening (r = 0.41) and velocity of circumferential fiber shortening (VCF) (r = 0.51). This study demonstrates that M-mode echocardiography is a very useful method for determining LV dimensions in children with normal LV volume, but is less accurate in children with left ventricular volume overload or with abnormal septal orientation or postoperative status after ventriculotomy.